Long-term outcome in a patient with pulmonary hypertension and hereditary hemorrhagic telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT) may be associated with pulmonary hypertension (PH). In the context that little attention has been given to long-term follow-up of such individuals, we report a patient with PH associated with HHT with special attention to clinical features and long-term response to therapy. To our knowledge, this case represents only the second with a 10-year follow-up reported and demonstrates that aggressive therapy can lead to long-term improvement in clinical parameters and survival.